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Childhood hearing loss

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https://www.readbyqxmd.com/read/28629849/analysis-of-archived-newborn-dried-blood-spots-dbs-identifies-congenital-cytomegalovirus-as-a-major-cause-of-unexplained-pediatric-sensorineural-hearing-loss
#1
Lucy Meyer, Bazak Sharon, Tina C Huang, Abby C Meyer, Kristin E Gravel, Lisa A Schimmenti, Elizabeth C Swanson, Hannah E Herd, Nelmary Hernandez-Alvarado, Kirsten R Coverstone, Mark McCann, Mark R Schleiss
PURPOSE: Congenital cytomegalovirus (cCMV) infection is the most common non-genetic cause of sensorineural hearing loss (SNHL). However, accurate diagnosis of cCMV as the etiology of SNHL is problematic beyond the neonatal period. This study therefore examined whether cCMV infection could be identified retrospectively in children presenting with unexplained SNHL to a multidisciplinary diagnostic outpatient otolaryngology clinic at an academic medical center in Minnesota. METHODS: Over a 4-year period, 57 patients with an age range of 3months to 10years with unexplained SNHL were recruited to participate in this study...
June 7, 2017: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/28609304/suprameatal-cochlear-implantation-in-a-charge-patient-with-a-novel-chd7-variant-and-kallmann-syndrome-phenotype-a-case-report
#2
Akira Ganaha, Tetsuya Tono, Tadashi Kaname, Kumiko Yanagi, Teruyuki Higa, Shunsuke Kondo, Hiroyuki Maeda, Mikio Suzuki
OBJECTIVE: We present the clinical findings, technique of the suprameatal cochlear implantation, postoperative auditory results, and genetic analysis of the CHD7 gene. PATIENT: A 19-year-old Japanese woman was referred because of progressive hearing loss since early childhood. She had used verbal language for the main mode of communication until the age of 17. Examination revealed coloboma, heart defect, choanal atresia, genital hypoplasia, and deafness, which was diagnosed as CHARGE syndrome...
June 12, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28607211/consensus-statement-of-the-indian-academy-of-pediatrics-on-newborn-hearing-screening
#3
Abraham Paul, Chhaya Prasad, S S Kamath, Samir Dalwai, M K C Nair, Waheeda Pagarkar
JUSTIFICATION: Hearing impairment is one of the most critical sensory impairments with significant social and psychological consequences. Reported incidence of congenital hearing loss is 30 per 10,000 children. Evidence-based, standardized national guidelines are needed for professionals to screen for hearing impairment during the neonatal period. PROCESS: The meeting on formulation of national consensus guidelines on developmental disorders was organized by Indian Academy of Pediatrics in Mumbai, on 18th and 19th December, 2015...
June 4, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28602134/the-effects-of-early-auditory-based-intervention-on-adult-bilateral-cochlear-implant-outcomes
#4
Stacey R Lim
OBJECTIVES: The goal of this exploratory study was to determine the types of improvement that sequentially implanted auditory-verbal and auditory-oral adults with prelingual and childhood hearing loss received in bilateral listening conditions, compared to their best unilateral listening condition. METHODS: Five auditory-verbal adults and five auditory-oral adults were recruited for this study. Participants were seated in the center of a 6-loudspeaker array. BKB-SIN sentences were presented from 0° azimuth, while multi-talker babble was presented from various loudspeakers...
June 12, 2017: Cochlear Implants International
https://www.readbyqxmd.com/read/28594466/bleeding-is-not-the-main-clinical-issue-in-many-patients-with-inherited-thrombocytopaenias
#5
REVIEW
F Melazzini, C Zaninetti, C L Balduini
Bleeding diathesis has been considered for a long time the main clinical issue impacting the lives of patients affected by inherited thrombocytopaenias. However, the number of known inherited thrombocytopaenias greatly increased in recent years, and careful evaluation of hundreds of patients affected by these 'new' disorders revealed that most of them are at risk of developing additional life-threatening disorders during childhood or adult life. These additional disorders are usually more serious and dangerous than low platelet count...
June 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28590156/hearing-loss-after-platinum-treatment-is-irreversible-in-noncranial-irradiated-childhood-cancer-survivors
#6
Eva Clemens, Andrica Ch de Vries, Antoinette Am Zehnhoff-Dinnesen, Wim Je Tissing, Jacqueline J Loonen, Saskia Fm Pluijm, Eline van Dulmen-den Broeder, Dorine Bresters, Birgitta Versluys, Leontien Cm Kremer, Helena J van der Pal, Sebastian Jccm Neggers, Martine van Grotel, Marry M van den Heuvel-Eibrink
Cisplatin and carboplatin are effective antineoplastic agents. They are also considered to be potentially highly ototoxic. To date, no long-term follow-up data from well-documented cohorts with substantial numbers of childhood cancer survivors (CCS) with platinum-related hearing loss are available. Therefore, in this study, we studied the reversibility of ototoxicity from discontinuation of treatment onwards in a national cohort of platinum-treated survivors with hearing loss at the end of cancer treatment...
June 7, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28573831/auditory-and-otologic-profile-of-alstr%C3%A3-m-syndrome-comprehensive-single-center-data-on-38-patients
#7
Spencer Lindsey, Carmen Brewer, Olga Stakhovskaya, Hung Jeffrey Kim, Chris Zalewski, Joy Bryant, Kelly A King, Jürgen K Naggert, William A Gahl, Jan D Marshall, Meral Gunay-Aygun
Alström syndrome (AS) is a rare autosomal recessive ciliopathy caused by mutations in the ALMS1 gene. Hallmark characteristics include childhood onset of severe retinal degeneration, sensorineural hearing loss, obesity, insulin-resistant diabetes, and cardiomyopathy. Here we comprehensively characterize the auditory and otologic manifestations in a prospective case series of 38 individuals, aged 1.7-37.9 years, with genetically confirmed AS. Hearing loss was preceded by retinal dystrophy in all cases, and had an average age of detection of 7...
June 1, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28522090/hearing-loss-mediates-executive-function-impairment-in-sleep-disordered-breathing
#8
Catherine M Hill, Romola S Bucks, Colin R Kennedy, Dawn Harrison, Annette Carroll, Nicolas Upton, Alexandra M Hogan
BACKGROUND: Sleep-disordered breathing (SDB) is often co-morbid with conductive hearing loss in early childhood due to a shared aetiology of adenotonsillar hypertrophy. Hearing loss is independently associated with impairment of executive function and behavioural difficulties. We hypothesised that these impairments in children with SDB may be mediated through hearing loss. METHODS: Fifty-eight children including 37 snorers awaiting adenotonsillectomy and 21 healthy non-snoring controls, aged 3-5 years, were assessed with pure tone audiometry, Strengths and Difficulties (SDQ), Behaviour Rating of Executive Function (BRIEF-P), and Childhood Middle Ear Disease and Hearing questionnaires...
June 2017: Sleep Medicine
https://www.readbyqxmd.com/read/28516959/adaptive-psychological-structure-in-childhood-hearing-impairment-audiological-correlations
#9
A Serra, G Spinato, S Cocuzza, L Licciardello, P Pavone, L Maiolino
The present research deals with the clinical and social problems present during linguistic and cognitive development of deaf children. Currently, the development of Theory of Mind represents an important research field in deafness studies. These international studies highlighted a significant alteration in the development of Theory of Mind in deaf children compared to normal hearing children, especially in cases of congenital or preverbal hearing loss. In particular, the research focuses on the skills of deaf children in recognising emotions and desires, through both perceptive and cognitive methods, by evaluation of psycho-cognitive skills of children with severe hearing loss using a set of questions to be administered to hearing loss patients...
June 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28513246/molecular-composition-and-distribution-of-gap-junctions-in-the-sensory-epithelium-of-the-human-cochlea-a-super-resolution-structured-illumination-microscopy-sr-sim-study
#10
Wei Liu, Hao Li, Fredrik Edin, Johan Brännström, Rudolf Glueckert, Annelies Schrott-Fischer, Matyas Molnar, Dirk Pacholsky, Kristian Pfaller, Helge Rask-Andersen
BACKGROUND: Mutations in the GJB2 gene, which encodes the Connexin26 (Cx26) protein, are the most common cause of childhood hearing loss in American and European populations. The cochlea contains a gap junction (GJ) network in the sensory epithelium and two connective tissue networks in the lateral wall and spiral limbus. The syncytia contain the GJ proteins beta 2 (GJB2/Cx26) and beta 6 (GJB6/Cx30). Our knowledge of their expression in humans is insufficient due to the limited availability of tissue...
May 17, 2017: Upsala Journal of Medical Sciences
https://www.readbyqxmd.com/read/28492720/association-between-language-and-hearing-disorders-risk-identification
#11
Alessandra Giannella Samelli, Silmara Rondon-Melo, Camila Maia Rabelo, Daniela Regina Molini-Avejonas
OBJECTIVE: To identify children at risk for hearing and/or language disorders and to investigate the association between these risks by conducting pre-validated hearing and language screenings. METHODS: The study was conducted during a polio vaccination campaign in August of 2013 in basic health units in western São Paulo. Parents of children between 2 and 5 years of age were asked to complete two screening tools: a hearing questionnaire (regarding hearing development) and a language production and comprehension scale (including the major language development milestones)...
April 2017: Clinics
https://www.readbyqxmd.com/read/28485286/assesment-effectiveness-of-treatment-eustachian-tube-dysfunction-using-pneumatic-inhaler-amsa
#12
COMPARATIVE STUDY
Iwona Zając-Ratajczak, Agata Szkiełkowska, Karina Wilhelmsen
INTRODUCTION: Upper respiratory tract infections are common childhood diseases. Children are more susceptible to middle ear infections because their Eustachian tube is short, straight and wide. As a consequence of these inflammatory changes while impaired patency of the Eustachian tube often arises conductive hearing. OBJECTIVE: The aim of this study was to Assessment effectiveness of treatment Eustachian tube dysfunction using inhaler AMSA. MATERIAL AND METHODS: The study group comprised 30 patients...
November 20, 2016: Otolaryngologia Polska
https://www.readbyqxmd.com/read/28483218/hearing-thresholds-and-ventilation-tube-treatment-in-children-with-unilateral-cleft-lip-and-palate
#13
Birgitta Tengroth, Christina Hederstierna, Erik Neovius, Traci Flynn
OBJECTIVE: Children with cleft lip and palate have a high prevalence of otitis media with effusion (OME) which is often associated with a fluctuating, conductive hearing loss in the low and mid-frequencies and a risk for permanent hearing loss in the higher frequencies. Although common, there is no consensus on the treatment of OME with ventilation tubes. The aim of this study is to document if the risk for permanent hearing loss and acquired cholesteatoma increases due to treatment with ventilation tubes (VT treatments) during childhood in a group of children with cleft lip and palate...
June 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28482824/novel-missense-mutation-in-the-bzip-transcription-factor-maf-associated-with-congenital-cataract-developmental-delay-seizures-and-hearing-loss-aym%C3%A3-gripp-syndrome
#14
Shari Javadiyan, Jamie E Craig, Shiwani Sharma, Karen M Lower, Theresa Casey, Eric Haan, Emmanuelle Souzeau, Kathryn P Burdon
BACKGROUND: Cataract is a major cause of severe visual impairment in childhood. The purpose of this study was to determine the genetic cause of syndromic congenital cataract in an Australian mother and son. METHOD: Fifty-one genes associated with congenital cataract were sequenced in the proband using a custom Ampliseq library on the Ion Torrent Personal Genome Machine (PGM). Reads were aligned against the human genome (hg19) and variants were annotated. Variants were prioritised for validation by Sanger sequencing if they were novel, rare or previously reported to be associated with paediatric cataract and were predicted to be protein changing...
May 8, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28479298/childhood-psychogenic-hearing-loss-identification-and-diagnosis
#15
M Parodi, I Rouillon, C Rebours, F Denoyelle, N Loundon
Psychogenic hearing loss, formerly known as functional or non-organic hearing loss, is a classic cause of consultation in infantile audiology. Risk factors include female gender, and age 8 or 12 years. Onset is relatively sudden, without impact on schooling or voice quality. Audiometric signs comprise non-superimposable audiometric thresholds (variable audiometric results), bilaterality, flat mean audiometric curve, and discrepancy between pure-tone and speech audiometry. The child needs reassuring during audiometric examination: attention-diversion techniques may be effective...
May 4, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/28475714/high-risk-factors-associated-with-early-childhood-hearing-loss-a-3-year-review
#16
Kelsey A Dumanch, Lenore Holte, Tammy O'Hollearn, Elizabeth Walker, Jacob Clark, Jacob Oleson
Purpose: In this study, we examined the association between risk factors for hearing loss and early childhood hearing status (normal hearing, congenital hearing loss, or delayed-onset hearing loss). Follow-up rates of audiologic care following passed or referred birth screens for children with risk factors were also examined. Method: A retrospective data review was completed on 115,039 children born from 2010 to 2012. Data analyses included prevalence rates, odds ratios, and Fisher exact tests of statistical significance...
June 13, 2017: American Journal of Audiology
https://www.readbyqxmd.com/read/28457383/kidney-transplantation-in-alstr%C3%A3-m-syndrome-case-report
#17
L Poli, G Arroyo, M Garofalo, E Choppin de Janvry, G Intini, A Saracino, R Pretagostini, F Della Pietra, P B Berloco
The Alström syndrome is a rare genetic disorder, inherited in an autosomal recessive manner. It has recently been classified as a ciliopathic disorder. Alström syndrome is a multiorgan pathology characterized by cone-rod dystrophy, hearing loss, childhood truncal obesity, insulin resistance and hyperinsulinemia, type 2 diabetes mellitus, dyslipidemia, short stature in adulthood, hypothyroidism, hypogonadism, dilated or restrictive cardiomyopathy, and progressive pulmonary, hepatic, and renal dysfunction. End-stage renal disease can occur as early as the late teens and is the leading cause of death...
May 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28399063/language-outcomes-in-deaf-or-hard-of-hearing-teenagers-who-are-spoken-language-users-effects-of-universal-newborn-hearing-screening-and-early-confirmation
#18
Hannah Pimperton, Jana Kreppner, Merle Mahon, Jim Stevenson, Emmanouela Terlektsi, Sarah Worsfold, Ho Ming Yuen, Colin R Kennedy
OBJECTIVES: This study aimed to examine whether (a) exposure to universal newborn hearing screening (UNHS) and b) early confirmation of hearing loss were associated with benefits to expressive and receptive language outcomes in the teenage years for a cohort of spoken language users. It also aimed to determine whether either of these two variables was associated with benefits to relative language gain from middle childhood to adolescence within this cohort. DESIGN: The participants were drawn from a prospective cohort study of a population sample of children with bilateral permanent childhood hearing loss, who varied in their exposure to UNHS and who had previously had their language skills assessed at 6-10 years...
April 10, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28384795/child-and-adolescent-health-from-1990-to-2015-findings-from-the-global-burden-of-diseases-injuries-and-risk-factors-2015-study
#19
Nicholas Kassebaum, Hmwe Hmwe Kyu, Leo Zoeckler, Helen Elizabeth Olsen, Katie Thomas, Christine Pinho, Zulfiqar A Bhutta, Lalit Dandona, Alize Ferrari, Tsegaye Tewelde Ghiwot, Simon I Hay, Yohannes Kinfu, Xiaofeng Liang, Alan Lopez, Deborah Carvalho Malta, Ali H Mokdad, Mohsen Naghavi, George C Patton, Joshua Salomon, Benn Sartorius, Roman Topor-Madry, Stein Emil Vollset, Andrea Werdecker, Harvey A Whiteford, Kalkidan Hasen Abate, Kaja Abbas, Solomon Abrha Damtew, Muktar Beshir Ahmed, Nadia Akseer, Rajaa Al-Raddadi, Mulubirhan Assefa Alemayohu, Khalid Altirkawi, Amanuel Alemu Abajobir, Azmeraw T Amare, Carl A T Antonio, Johan Arnlov, Al Artaman, Hamid Asayesh, Euripide Frinel G Arthur Avokpaho, Ashish Awasthi, Beatriz Paulina Ayala Quintanilla, Umar Bacha, Balem Demtsu Betsu, Aleksandra Barac, Till Winfried Bärnighausen, Estifanos Baye, Neeraj Bedi, Isabela M Bensenor, Adugnaw Berhane, Eduardo Bernabe, Oscar Alberto Bernal, Addisu Shunu Beyene, Sibhatu Biadgilign, Boris Bikbov, Cheryl Anne Boyce, Alexandra Brazinova, Gessessew Bugssa Hailu, Austin Carter, Carlos A Castañeda-Orjuela, Ferrán Catalá-López, Fiona J Charlson, Abdulaal A Chitheer, Jee-Young Jasmine Choi, Liliana G Ciobanu, John Crump, Rakhi Dandona, Robert P Dellavalle, Amare Deribew, Gabrielle deVeber, Daniel Dicker, Eric L Ding, Manisha Dubey, Amanuel Yesuf Endries, Holly E Erskine, Emerito Jose Aquino Faraon, Andre Faro, Farshad Farzadfar, Joao C Fernandes, Daniel Obadare Fijabi, Christina Fitzmaurice, Thomas D Fleming, Luisa Sorio Flor, Kyle J Foreman, Richard C Franklin, Maya S Fraser, Joseph J Frostad, Nancy Fullman, Gebremedhin Berhe Gebregergs, Alemseged Aregay Gebru, Johanna M Geleijnse, Katherine B Gibney, Mahari Gidey Yihdego, Ibrahim Abdelmageem Mohamed Ginawi, Melkamu Dedefo Gishu, Tessema Assefa Gizachew, Elizabeth Glaser, Audra L Gold, Ellen Goldberg, Philimon Gona, Atsushi Goto, Harish Chander Gugnani, Guohong Jiang, Rajeev Gupta, Fisaha Haile Tesfay, Graeme J Hankey, Rasmus Havmoeller, Martha Hijar, Masako Horino, H Dean Hosgood, Guoqing Hu, Kathryn H Jacobsen, Mihajlo B Jakovljevic, Sudha P Jayaraman, Vivekanand Jha, Tariku Jibat, Catherine O Johnson, Jost Jonas, Amir Kasaeian, Norito Kawakami, Peter N Keiyoro, Ibrahim Khalil, Young-Ho Khang, Jagdish Khubchandani, Aliasghar A Ahmad Kiadaliri, Christian Kieling, Daniel Kim, Niranjan Kissoon, Luke D Knibbs, Ai Koyanagi, Kristopher J Krohn, Barthelemy Kuate Defo, Burcu Kucuk Bicer, Rachel Kulikoff, G Anil Kumar, Dharmesh Kumar Lal, Hilton Y Lam, Heidi J Larson, Anders Larsson, Dennis Odai Laryea, Janni Leung, Stephen S Lim, Loon-Tzian Lo, Warren D Lo, Katharine J Looker, Paulo A Lotufo, Hassan Magdy Abd El Razek, Reza Malekzadeh, Desalegn Markos Shifti, Mohsen Mazidi, Peter A Meaney, Kidanu Gebremariam Meles, Peter Memiah, Walter Mendoza, Mubarek Abera Mengistie, Gebremichael Welday Mengistu, George A Mensah, Ted R Miller, Charles Mock, Alireza Mohammadi, Shafiu Mohammed, Lorenzo Monasta, Ulrich Mueller, Chie Nagata, Aliya Naheed, Grant Nguyen, Quyen Le Nguyen, Elaine Nsoesie, In-Hwan Oh, Anselm Okoro, Jacob Olusegun Olusanya, Bolajoko O Olusanya, Alberto Ortiz, Deepak Paudel, David M Pereira, Norberto Perico, Max Petzold, Michael Robert Phillips, Guilherme V Polanczyk, Farshad Pourmalek, Mostafa Qorbani, Anwar Rafay, Vafa Rahimi-Movaghar, Mahfuzar Rahman, Rajesh Kumar Rai, Usha Ram, Zane Rankin, Giuseppe Remuzzi, Andre M N Renzaho, Hirbo Shore Roba, David Rojas-Rueda, Luca Ronfani, Rajesh Sagar, Juan Ramon Sanabria, Muktar Sano Kedir Mohammed, Itamar S Santos, Maheswar Satpathy, Monika Sawhney, Ben Schöttker, David C Schwebel, James G Scott, Sadaf G Sepanlou, Amira Shaheen, Masood Ali Shaikh, June She, Rahman Shiri, Ivy Shiue, Inga Dora Sigfusdottir, Jasvinder Singh, Naris Silpakit, Alison Smith, Chandrashekhar Sreeramareddy, Jeffrey D Stanaway, Dan J Stein, Caitlyn Steiner, Muawiyyah Babale Sufiyan, Soumya Swaminathan, Rafael Tabarés-Seisdedos, Karen M Tabb, Fentaw Tadese, Mohammad Tavakkoli, Bineyam Taye, Stephanie Teeple, Teketo Kassaw Tegegne, Girma Temam Shifa, Abdullah Sulieman Terkawi, Bernadette Thomas, Alan J Thomson, Ruoyan Tobe-Gai, Marcello Tonelli, Bach Xuan Tran, Christopher Troeger, Kingsley N Ukwaja, Olalekan Uthman, Tommi Vasankari, Narayanaswamy Venketasubramanian, Vasiliy Victorovich Vlassov, Elisabete Weiderpass, Robert Weintraub, Solomon Weldemariam Gebrehiwot, Ronny Westerman, Hywel C Williams, Charles D A Wolfe, Rachel Woodbrook, Yuichiro Yano, Naohiro Yonemoto, Seok-Jun Yoon, Mustafa Z Younis, Chuanhua Yu, Maysaa El Sayed Zaki, Elias Asfaw Zegeye, Liesl Joanna Zuhlke, Christopher J L Murray, Theo Vos
Importance: Comprehensive and timely monitoring of disease burden in all age groups, including children and adolescents, is essential for improving population health. Objective: To quantify and describe levels and trends of mortality and nonfatal health outcomes among children and adolescents from 1990 to 2015 to provide a framework for policy discussion. Evidence Review: Cause-specific mortality and nonfatal health outcomes were analyzed for 195 countries and territories by age group, sex, and year from 1990 to 2015 using standardized approaches for data processing and statistical modeling, with subsequent analysis of the findings to describe levels and trends across geography and time among children and adolescents 19 years or younger...
June 1, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28372531/panel-1-epidemiology-and-diagnosis
#20
Preben Homøe, Kari Kværner, Janet R Casey, Roger A M J Damoiseaux, Thijs M A van Dongen, Hasantha Gunasekera, Ramon G Jensen, Ellen Kvestad, Peter S Morris, Heather M Weinreich
Objective To create a literature review between 2011 and June 1, 2015, on advances in otitis media (OM) epidemiology and diagnosis (including relevant audiology studies). Data Sources Electronic search engines (PubMed, EMBASE, and Cochrane Library) with a predefined search strategy. Review Methods Articles with appropriate epidemiologic methodology for OM, including acute mastoiditis and eustachian tube dysfunction. Items included OM worldwide and in high-risk populations, OM-related hearing loss, news in OM diagnostics, prenatal risk factors and comorbidities, postnatal risk factors, genetics, microbiological epidemiology, guidelines, and quality of life...
April 2017: Otolaryngology—Head and Neck Surgery
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